A Very Rare Case of De Novo Histoid Leprosy with Type 1 Reaction

Aninda Marina

, Sondang P. Sirait

, Sri Linuwih Menaldi

Department of Dermatology and Venereology Faculty of Medicine Universitas Indonesia/Dr. Cipto Mangunkusumo

National Central General Hospital, Indonesia

Corresponding author

Keywords: Histoid, De Novo, Leprosy, Reversal Reaction

Abstract: Histoid Leprosy (HL) is a distinct and rare type of leprosy with unique clinical manifestations and specific

histopathology of multibacillary leprosy (MB). Histoid leprosy generally occurs in lepromatous leprosy

patients (LL) or borderline lepromatous (BL). Often it occurs after inadequate dapsone treatment despite

sometimes arise de novo as well. Histoid forms can ensue accompanied by reactions of erythema nodosum

leprosum (ENL) and rarely reversal reactions. Diagnosis of HL based on clinical manifestations,

bacteriological examination, and histopathological examination. Clinical manifestations are typical such as

coppery nodules, well-defined, might be soft or hard, shiny, with varying sizes. Histopathological findings

showed a longitudinal or spindle histiocyte containing M. leprae. We report a rare case of histoid leprosy

accompanied by reversal reactions. A 59-year-old Chinese woman, from West Borneo, complained of a

painless nodule on the face and body since two months ago with swollen face and rashes. Patients never had

leprosy treatment before Histopathological shows some spindle cells. Patients are diagnosed with histoid

leprosy and reversal reactions. Diagnosis of histoid leprosy is made by history, physical examination, and

histopathology. Patients fall into the de novo category due to having never been treated with dapsone before.

The therapy given to patients is multibacillary multi drugs therapy (MDT-MB) and corticosteroids. The

patient showed improvement in lesions after six months of MDT-MB and corticosteroids for three months.

Histoid leprosy is able to occur de novo and accompanied by reversal reactions. Early diagnosis and

complete treatment are crucial to achieve the goal of elimination of leprosy.

1 INTRODUCTION

Histoid leprosy (HL) is a rare type of leprosy with

typical and histopathological clinical manifestations

specific to the multibacillary type of leprosy (MB).

Wade in 1960 first reported the form of leprosy, and

several other cases in 1963. (Sehgal VN, 2016)

Histoid leprosies generally occurs in lepromatous

(LL) or borderline lepromatous (BL) type leprosy

without or during or after treatment dapsone.

1,2

Some reports also show that HL could manifest in

paucibacillary type (PB).(Sehgal VN, 2006; Rao

AG, 2016)

Additionally, this histoid can also occur

in patients with severe immunosuppression

conditions caused by human immunodeficiency

virus (HIV) or even with erythema nodosum

leprosum (ENL) reactions and reversal

reactions(Sun J et al, 2017; Kolaparambath BA et al,

2014).

HL incidence in India is around 2.79-3.60% of

all leprosy patients, and de novo incidents are

increasing every day.(Kaur et al., 2009; Hali F et al

2011). There is still an unknown number of incidents

in Indonesia. Men are dominant to women and rarely

occur in children. Histoid leprosy is a variant of LL

type, but the HL immune response is better both

cellular and humoral immunity. .(Sehgal VN, 2016)

HL diagnosis based on clinical manifestations,

bacteriological examination, and histopathological

examination. (Sehgal VN, 2016;Kalla G et al.,

2000).

Clinical manifestations HL is very typical in the

form of coppery nodules, well-defined, can be soft

or hard, shiny, with varying sizes (diameter up to 3

cm) that arise in cutaneous or subcutaneous above

normal-looking skin. The predilection of lesions

usually on the face, arms, back, chest, abdomen, and

buttocks. Bacteriological examination results show

an increase of solid bacilli due to the multiplication

of M. leprae experiencing resistance, which causes

312

Marina, A., Sirait, S. and Menaldi, S.

A Very Rare Case of De Novo Histoid Leprosy with Type 1 Reaction.

DOI: 10.5220/0009987603120315

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 312-315

ISBN: 978-989-758-469-5

bacteriological index (IB) and morphological index

(IM) levels high. .(Sehgal VN, 2016)

Histopathological findings HL shows histiocytes

that are elongated or spindle-shaped cells containing

M. leprae, thus forming a curved arrangement.

Treatment of histoid leprosy uses multibacillary

multidrug therapy (MDT-MB), consisting of

rifampicin, dapsone, and clofazimine given for at

least two years or up to a negative morphological

index.

2 CASE

A 50 years old woman, Chinese descendants from

Pemangkat West Borneo, came to the dermato-

venereology clinic of Cipto Mangunkusumo

National General Hospital with chief complain of

painless nodules on her face and body since two

months ago. Initially four months ago patient

complained of red patches on both leg, and buttocks.

The patches felt numb and widespread gradually.

Patients were tested for acid-resistant bacilli (AFB)

and blood tests in other hospitals, and the result was

positive for leprosy. She was given multidrug

multibacillary therapy for leprosy with a target of

1year therapy.

After the second month treatment, she has

multiple erythema nodules appeared on the face,

body, arms, and legs. Nodulesare painless with

minimal pruritus. Nodules are expanding and

multiply. The patient feels uncomfortable with a

nodule on her face due to disturbing appearance.

There is no fever nor joint pain. There are no lesions

that become increasingly numb or weak. There are

no complaints of thinning eyebrows nor dry

skin.The patient also complained about swollen face

and both legs with more erythema on the patches

and nodules in the last one month. The patient is

treated with 3 x 4 mg methylprednisolone. Swelling

is reduced. But the erythema is still the same. There

is no radiating nerve pain, and there are no new

patches. History of taking drugs other than leprosy is

denied in the past two months. There was a history

of drinking herbal medicine two months ago. She

never takes any leprosy drug before. No one in her

family or neighbor has ever had leprosy that she

knew.

Physical examination shows multiple painless

erythematous papules to nodules, some of it was

skin-colored, dome-shaped, on the face, chest, arms,

trunk, abdomen, and legs. There also an

erythematous plaque on both legs and buttocks, well

defined, and some of it has some punch out-like

lesion. Non-pitting edema found on the face and

foot. There was enlargement without tender on both

Nerve Peroneus communis and N.Tibialis posterior.

Skin smear examination shows no solid bacilli but

positive fragmented bacilli (+2 Bacteriological

Index) on both ears.

Histopathology examination shows Grenz zone,

circumscribed macrophage granuloma with the

predominance of spindle-shaped cells, foreign body

giant cells, and some foamy macrophages. Some of

the cells look edema. Fite Faraco shows no acid-fast

bacilli. All the examination leads to histoid leprosy

with reversal reaction for diagnosis. The patient is

treated with WHO regimen multidrug therapy

multibacillary such as rifampicin 600mg,

clofazimine 300mg, and dapsone 100mg once a

month with dapsone 100mg and clofazimine 50mg

daily for two years. The patient also got

methylprednisolone 32mg daily (equivalent

prednisone 0.8mg/kg) then gradually reduced

weekly and eventually stopped for reversal reaction.

Clinical improvements both subjective, and

objective in six months of multidrug multibacillary

therapy and three months of corticosteroid therapy.

3 DISCUSSION

Histoid leprosy (HL) is a rare type of leprosy with

typical and histopathological clinical manifestations

specific to the multibacillary type of leprosy (MB).

.(Sehgal VN, 2016)

It is characterized by unique

clinical, histopathological, and microbiological

features. Histoid leprosy generally occurs in

lepromatous (LL) or borderline lepromatous (BL)

type leprosy without or during or after treatment

dapsone. This form of leprosy is relatively common

in patients on dapsone monotherapy and irregular

treatment. Sometimes, it can arise de novo as well.

.(Sehgal VN, 2016;Alious VN, 2006). Some reports

also show that HL could manifest in paucibacillary

type (PB) .(Sehgal VN, 2006,Rao AG, 2016).

Additionally, this histoid can also occur in patients

with severe immunosuppression conditions caused

by human immunodeficiency virus (HIV) or even

with erythema nodosum leprosum (ENL) reactions

and reversal reactions.(Sun J et al., 2017; Kaur, et al,

2009).

There is male preponderance, rarely in children,

and the average age at diagnosis is between 21 and

40 years. Clinically, it is characterized by cutaneous

or subcutaneous nodules and papules, which are

painless, coppery nodules, well-defined, can be soft

or hard, shiny, with varying sizes (diameter up to 3

A Very Rare Case of De Novo Histoid Leprosy with Type 1 Reaction

313

cm) that arise in cutaneous or subcutaneous above

normal-looking skin. The lesions are usually located

on the posterior and lateral aspects of the arms,

buttocks, thighs, dorsum of the hands, lower part of

the back, and over the bony prominences, especially

over elbows and knees. (Sehgal VN, 2016).

Clinically, histoid leprosy may mimic lepromatous

leprosy or ENL reaction.

However, specific histopathology of histoid

leprosy differentiates it from LL type which shows

macrophage granuloma with a variable amount of

foamy changes with many bacilli and globi and ENL

reaction which shows features of acute inflammation

predominantly having neutrophils accompanied by

edema along with granular AFB. Histoid leprosy

might represent an enhanced response of the

multibacillary disease in localizing the disease

process. An increase in both cell-mediated and

humoral immunity against Mycobacterium leprae, as

in lepromatous leprosy, has been hypothesized.

From history, the patient's gender and age were

not in accordance with the epidemiological data on

the incidence of the most common histoid leprosy.

However, epidemiological data obtained from India,

not Indonesia.(Kaur et al., 2009). She is a Chinese

descendant which are quite susceptible to

leprosy.

Patients had never taken leprosy treatment

before which is defined as "de novo".(Pandey P et al,

2015). The patient complained of swelling, old

reddening patches, and a history of drinking herbs.

This is in accordance with the reversal reaction. In

some cases, a reversal reaction with histoid leprosy

has been found simultaneously. (Sun J et al.,

2017;Singh N et al., 2015).On physical examination,

face, chest, arms bilaterally, back, abdomen, and

limbs shows multiple erythematous-skin-colored

nodules, dome-shaped, shiny, milliary-nummular

size. There is no tenderness. Predilection and

morphology of the lesions are in accordance with the

appearance of histoid leprosy but can still be

diagnosed in comparison with MH

nodularlepromatous type.

Clinical differences

between nodular lepromatous type and HL are in

nodular lepromatous type, and nodules arise from

infiltrated regions while in HL from healthy skin.

The nodular lepromatous type has diffuse nodes

whereas well defined in the histoid node.

The differential diagnosis, which is erythema

nodosum lepromatous, can be excluded because

there is no tenderness and no history of pain.

Erythematous plaques on the buttocks and limbs,

well defined, with punch-out like lesion

accompanied with a dry white patch above them and

hypoesthesia, are thought to be borderline

tuberculoid lesions. Edema and warmth on plaque

palpation are thought to be due to reversal reactions.

Nerve enlargement is found in both N. peroneus

communis and posterior tibialis. Investigation of slit

skin smear was obtained, and the result is +2 in the

bacterial index from right ear lesions. This indicates

that the patient belongs to the multibacillary type.

AFB results were only found in a few fragmented

bacilli that were not compatible with histoid leprosy.

However, in some cases, AFB is only positive in

histoid lesions, not in skin lesions that look normal.

This is what distinguishes lepromatous leprosy.

It is

necessary to do a histopathological examination to

determine the type of leprosy further.

Histopathological examination with hematoxylin-

eosin (HE) staining was obtained, and it shows

Grenz zone, macrophage granuloma with foam cells,

and a lot of spindle cells. HL specific

histopathological features, namely the depletion of

the epidermis due to the pressure of the dermal

pseudocapsule mass consisting of histiocytes

consisting of spindles and intertwining. It is different

from lepromatous histopathology, which is thinning

of the epidermis, the rete ridge becomes flatter, the

grenz zone, and the dermis is found diffuse leproma

consisting of foamy macrophages and lymphocytes

and plasma cells. Spindle cells are pathognomonic

markers in histoid histopathology. Fite-Faraco found

no AFB. From these results, it can be concluded that

in accordance with histoid leprosy despite there is no

AFB in Fite-Faraco staining.(Sehgal VN, 2016)

This could be caused by the low sensitivity of Fite-

Faraco staining (40-70%), and it worsens when the

bacterial index is below 3.(Cabic E, 2018;Adiga et

al., 2016)

In this case, MDT-MB therapy was given, which

provided clinical improvement after six months of

treatment. This is in accordance with the study of

Hali et al., Who evaluated treatment responses in

two HL patients and received improvement after

three months of providing MDT-MB.

Patients still

need MDT-MB for at least the next 18

months.

Patients were also given 32 mg /day of

Methylprednisolone (equivalent to Prednisone 40

mg/day) for two weeks then tapered off every two

weeks for 12 weeks to treat the reversal reaction.

Reversal reactions in patients have been completely

gone after giving 12 weeks. The prognosis in this

patient is Bonam because there are no complications

in the patient, and there are no other systemic

diseases. The function of patients isdubia ad bonam

due to possible improvement in nerve function in the

hands and feet due to leprosy, according to these

patients, there is no sensory or motor impairment.

ICTROMI 2019 - The 2nd International Conference on Tropical Medicine and Infectious Disease

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This patient needs to be examined but must wait at

least once a month. Prognosis and sanactionam are

dubia ad bonam because histoid leprosy can be

cured if taking medication regularly and completing

treatment for two years but can relapse again while

getting treatment or after complete treatment.

4 CONCLUSION

Histoid leprosy is a rare type of leprosy and the

diagnosis made by history, physical examination,

and histopathology. Patients fall into the de novo

category due to having never been treated with

dapsone before. The therapy given to patients is

multibacillary multi drugs therapy (MDT-MB) and

corticosteroids. The patient showed improvement in

lesions after six months of MDT-MB and

corticosteroids for three months. Histoid leprosy is

able occurred de novo and accompanied by reversal

reactions despite its very rare. This case may act as

reservoirs of the disease and lead to further spread of

leprosy. Early diagnosis and complete treatment are

crucial to achieve the goal of elimination of leprosy.

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